It is usually inherited (known as familial idiopathic basal ganglia calcification) most commonly as an autosomal dominant trait, but it may also occur sporadically.[1]

The age of onset is usually in the third and fourth decades. The clinical picture is heterogeneous and includes neurological and neuropsychiatric symptoms. Movement disorders are usually the prominent neurological sign and they include a variety of conditions such as parkinsonism, chorea, tremors, dystonia, and athetosis.[2] Neuropsychiatric symptoms range from difficulty with concentration and memory to changes in personality or behaviors to psychosis and dementia. Seizures and signs of cerebellar dysfunction such as dysarthria and ataxia may also occur.[3]

The CT scan of the brain is sensitive enough to localize and assess the extent of cerebral calcification. Other investigations include blood and urine tests (such as serum concentrations of calcium, phosphorus, magnesium, alkaline phosphatase, calcitonin, and PTH).[4]

Fhar's syndrome is a neurodegenerative and progressive disorder and there is no treatment at this time.[5]

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